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Localised vulva Langerhans cell histiocytosis
Hikaru Ishigaki1, Naohito Hatta, Mizuki Yamada
1Department of Dermatology, Kanazawa University School of Medicine, 13-1 Takaramachi, Kanazawa, 920-8641 Japan.
European Journal of Dermatology : EJD
|November 27, 2004
Summary
Adult Langerhans cell histiocytosis (LCH) presenting solely as a skin lesion is uncommon. This case highlights vulvar ulcers as the only symptom in a 65-year-old female, successfully treated with surgery.
Area of Science:
- Dermatology
- Oncology
- Pathology
Background:
- Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder of Langerhans cells.
- While LCH commonly affects children, adult cases are less frequent and can present with diverse manifestations.
- Cutaneous involvement as the sole presentation of adult LCH is particularly rare.
Observation:
- A 65-year-old female presented with chronic vulvar ulcers.
- Histopathological examination revealed a diffuse proliferation of histiocytic cells.
- Immunohistochemistry confirmed positivity for S-100 protein and CD1a, characteristic markers for LCH.
Findings:
- Electron microscopy identified Birbeck granules within the cytoplasm of the lesional cells, further supporting the diagnosis of LCH.
- The patient had no evidence of systemic or other organ involvement.
- Complete surgical excision of the vulvar lesion was performed.
Implications:
- This case underscores the importance of considering LCH in adult patients with unusual cutaneous lesions, even in the absence of systemic symptoms.
- Complete surgical excision can be an effective treatment for localized adult cutaneous LCH, as evidenced by the 1-year follow-up without recurrence.
- Further research into the specific presentation and management of adult cutaneous LCH is warranted.